Porokeratosis palmariset plantaris disseminata - a rare entity

Porokeratosis is a well-known keratinization disorder where cornoid lamella is characteristically seen. There are seven clinical variants of which porokeratosis palmaris et plantaris disseminata [PPPD] is a very rare entity. Our PPPD case was sporadic and diagnosed early at 35 years of age. The lesions started on trunk and disseminated to extremities, face, oral cavity, palms and soles. Involvement of oral mucosa made easier to differentiate our case from disseminated superficial porokeratosis. The response was good with isotretinoin therapy

Familial leiomyomatosis cutis affecting nine family members in two successive generations including four cases of Reed's syndrome.

Reed's syndrome or familial leiomyomatosis cutis et uteri is an autosomal dominant disorder, characterized by multiple cutaneous and uterine leiomyomas. We report here a case of a 53-year-old woman who presented to us with multiple painful nodules over different parts of her body. Based on the histopathological examination, imaging, and past medical records, a diagnosis of Reed's syndrome was made. Three of her sisters had similar disease. Subsequently, it was found that a total of nine members of their family in two successive generations were affected with cutaneous leiomyomas. The present case series has been reported for its interesting clinical presentations and rarity.

Evening primrose oil is effective in atopic dermatitis: a randomized placebo-controlled trial.

BACKGROUND: Atopic dermatitis (AD) is a chronic, relapsing, itchy dermatosis of multifactorial origin, which commonly starts in childhood. Defective metabolism of essential fatty acids leading to relative dominance of pro-inflammatory prostaglandins (PGE2 and PGF2) has been reported as an important factor in the pathogenesis of AD. Evening primrose oil (EPO) as a source of gamma-linolenic acid (GLA) has been of interest in the management of AD. AIM: To evaluate the efficacy and safety of EPO in atopic dermatitis in our patients. METHODS: Consecutive new out-patient department (OPD) patients of a referral hospital in Kolkata clinically diagnosed as having AD were randomly allocated to two groups. To the first group, evening primrose oil was supplied as 500-mg oval clear unmarked capsules, while placebo capsules identical in appearance and containing 300 mg of sunflower oil were given to the other group. Treatment continued for a period of 5 months. With pre-designed scoring system (based on four major parameters: extent, intensity, itching, and dryness), clinical evaluation was done at baseline and subsequent monthly visits. Data of the first 25 patients from each group who completed the 5 months of trial were compiled and analyzed. RESULTS: At the end of the fifth month, 24 (96%) patients of EPO group and 8 (32%) patients of placebo group showed improvement. There was significant difference in outcome of treatment between two groups (P<0.00001). No significant adverse effect was reported by any patient/guardian at any point of assessment. CONCLUSION: Evening primrose oil is a safe and effective medicine in management of AD. However, since not all researchers across the world have found the same good result, further large trials on Indian patients are needed.